Primary pulmonary hypertension, as the name suggests, has no known cause.

Progression of Pulmonary Heart Disease – Cor Pulmonale

This depends on the aetiology (origins) of the underlying cause of the pulmonary hypertension. In cases of pulmonary embolism, one or many embolic clots (usually from the deep veins of the leg) occlude a part of the pulmonary arterial vasculature. In time, this results in increased pulmonary pressure, decreased cardiac output and a sydrome of right heart failure, usually with no cardiac compensation.

In the chronic setting (as in COPD) the pulmonary hypertension is due to changes to the lung tissues themselves. This is often associated with compensatory right heart changes, and eventually right heart failure.

How is Pulmonary Heart Disease – Cor Pulmonale Diagnosed?

1) Chest x-ray: may show evidence of right ventricular hypertrophy or atrial dilatation in the chronic setting. Prominent pulmonary arteries may be seen. May also show pulmonary effusions or infarctions secondary to embolism.
2) ECG: can demonstrate right ventricular hypertrophy and ischaemia. Tall tented p-pulmonale may also be seen.
3) Echocardiography may show dilatation and also allows indirect measurement of pulmonary artery pressures.

Prognosis of Pulmonary Heart Disease – Cor Pulmonale

The prognosis of pulmonary heart disease depends on the underlying causes. However, disease serious enough to cause significant right heart failure (usually massive pulmonary embolism or end stage COPD) are associated with poor prognoses.

Mild to moderate pulmonary emboli have the best outlook. Primary pulmonary hypertension has a poor prognosis with a porgressive course – heart and lung transplantation is an option in younger patients.

How is Pulmonary Heart Disease – Cor Pulmonale Treated?

Right heart failure secondary to pulmonary pathology should be treated similarly to congestive heart failure. Vasodilators such as the calcium channel blockers may be particularly useful in reducing the pressure of the pulmonary circulation.